Table 7.

Recommended Surveillance for Individuals with Mucopolysaccharidosis Type III

System/ConcernEvaluationFrequency
Neurologic Monitor treatment effectiveness in those w/seizures.As clinically indicated
Development Monitor developmental capabilities & educational needs.Annually
Psychiatric/
Behavioral issues
Assessment of destructive or disruptive behaviorsAs clinically indicated
Musculoskeletal
  • Monitor joint mobility; assessment by orthopedist.
  • Monitor BMD w/DXA & vitamin D metabolism studies.
Hearing Assessment by audiologist & otolaryngologistAnnually
Cardiovascular Echocardiogram & EKG to assess for valvular disease & conduction system defects
Ocular Ophthalmologic assessmentAs clinically indicated

BMD = bone mineral density; DXA = dual-energy x-ray absorptiometry

From: Mucopolysaccharidosis Type III

Cover of GeneReviews®
GeneReviews® [Internet].
Adam MP, Feldman J, Mirzaa GM, et al., editors.
Seattle (WA): University of Washington, Seattle; 1993-2024.
Copyright © 1993-2024, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved.

GeneReviews® chapters are owned by the University of Washington. Permission is hereby granted to reproduce, distribute, and translate copies of content materials for noncommercial research purposes only, provided that (i) credit for source (http://www.genereviews.org/) and copyright (© 1993-2024 University of Washington) are included with each copy; (ii) a link to the original material is provided whenever the material is published elsewhere on the Web; and (iii) reproducers, distributors, and/or translators comply with the GeneReviews® Copyright Notice and Usage Disclaimer. No further modifications are allowed. For clarity, excerpts of GeneReviews chapters for use in lab reports and clinic notes are a permitted use.

For more information, see the GeneReviews® Copyright Notice and Usage Disclaimer.

For questions regarding permissions or whether a specified use is allowed, contact: ude.wu@tssamda.

NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.